ANCA vasculitis in the elderly

Background: In population-based estimates of disease, the incidence of ANCA-associated vasculitides is increasing, with the peak age of onset in middle aged and older adults. Clinical characteristics of the very elderly have not been fully elucidated. Methods: Patients who met the criteria for Wegener granulomatosis and microscopic polyangiitis were included in the study. The following characteristics were analyzed by retrospective review: time interval from first symptoms to date of diagnosis, age at diagnosis, laboratory values on initial presentation, and Birmingham Vasculitis Activity Score when diagnosed. Results: Patients >75 years old were more often female, had fewer ear, nose, or throat symptoms, and had lower hemoglobin values. At date of latest follow-up, 40% of the older patients died compared with 11% of the younger cohort (P = 0.0006). Of the deceased elderly patients, nearly one-half died within 6 months. Age >75 carried an elevated risk of mortality (hazard ratio = 2.69, 95% confidence interval = 1.55-4.96; P = 0.0005) as did elevated serum creatinine (hazard ratio = 1.25 per 1 mg/dL, 95% confidence interval = 1.06-1.43; P = 0.0109). Survival was worse in those presenting with Birmingham Vasculitis Activity Score >20 and those >75-year-old. Conclusion: The clinical presentation of ANCA-associated vasculitis is relatively similar among elderly and younger patients. In patients aged 75 years or older, ANCA vasculitis is associated with higher mortality, and related to the presence of renal involvement. Elderly patients have a greater risk for death within the first 6 months after diagnosis.